Synchronous gnathic osteosarcoma and B-cell lymphoblastic lymphoma/leukemia: A rare case presentation.

Multiple primary malignancies are a well-recognized entity, with increased recognition and detection alongside development of hybrid imagining. We present a rare case of a 16-year-old male with gnathic osteosarcoma and incidental finding of a second silent synchronous B-cell lymphoblastic lymphoma/leukemia in the lower limb. Treated successfully by chemotherapy, radiotherapy, and surgery.

Polatuzumab Vedotin in a Patient with Refractory Burkitt Lymphoma, a Case Report.

Although Burkitt lymphoma is considered a curable disease due to the progress made in choosing the most effective first-line therapy, relapsed or refractory Burkitt lymphoma (BL) has a very poor outcome. There is a lack of data supporting the treatment regimens. We report a 48-year-old male with stage II Burkitt's lymphoma with no response to the first line of high-intensity chemotherapy. However, treatment with polatuzumab vedotin led to complete clinical remission for more than one year.

Myeloid Sarcoma Mimicking Dental Abscess in a Patient with Chronic Myeloid Leukemia: Diagnostic and Therapeutic Dilemma.

Chronic myeloid leukemia (CML) is a hematologic malignancy that has significant improvement in its prognosis after the introduction of tyrosine kinase inhibitors. Transformation to accelerated phase or blast phase can happen. Myeloid sarcoma or chloroma is an uncommon extramedullary disease. It is very unusual for patients with CML to develop myeloid sarcoma. We report a young man with CML in the chronic phase who developed myeloid sarcoma. There were many difficulties in the diagnosis of myeloid sarcoma due to the simulation of other more common conditions like infections and other malignancies. In addition, there are treatment challenges because of lack of standardized treatment. The case shed light on this rare complication, the challenging diagnosis, and its implication in patients with CML.

Leptomeningeal Metastatic L858R EGFR-mutant Lung Cancer: Prompt Response to Osimertinib in the Absence of T790M-mutation and Effective Subsequent Pulsed Erlotinib.

Leptomeningeal carcinomatosis (LMC) is a known sequel of metastatic lung cancer and its treatment is challenging. Nevertheless, treatment options for LMC due to metastatic epidermal growth factor receptor-mutant (EGFR-mutant) lung adenocarcinoma are expanding. We present a 52-year-old male patient with metastatic non-small-cell lung cancer (NSCLC). The patient was found to have L858R mutation in exon 21 of the EGFR gene. He was initially treated with erlotinib, followed by afatinib/cetuximab, followed by chemotherapy. Thereafter, his disease progressed to LMC. Although tissue biopsy did not show T790M-mutation, osimertinib (160 mg once daily) promptly induced clinical and radiological response that continued for five months. High dose pulsed erlotinib (1500 mg weekly) improved his quality of life and extended his survival for a further four months.

Gallium-68 Prostate-Specific Membrane Antigen Positron Emission Tomography: A Practical Guide for Radiologists and Clinicians.

Prostate cancer (PCa) is the third most common form of cancer and the most common cancer diagnosis in men in the United States. It is known that prostate-specific membrane antigen (PSMA) is overexpressed in PCa. PSMA is a type II transmembrane glycoprotein expressed in several benign and malignant tissues. In December 2020, FDA approved Gallium-68 (68Ga) PSMA, which is a PSMA-targeted positron emission tomography (PET) imaging agent. Molecular imaging targeting PSMA has shown substantial advancement in PCa imaging. In this article, we discuss the radiopharmaceutical, indications to do PSMA PET, technical aspects of PSMA PET imaging, normal biodistribution of PSMA, other benign and malignant conditions that can take up PSMA, staging of prostate cancer, and how to report PSMA PET.

Assessing Bone Marrow Activity with [18F]FLT PET in Patients with Essential Thrombocythemia and Prefibrotic Myelofibrosis: A Proof of Concept.

Objectives: This study aims to assess the value of FLT-PET as a non-invasive tool to differentiate between patients with ET and Pre-PMF. This study is a pilot study to have a proof of concept only. Methods: This is a prospective, interventional study where a total of 12 patients were included. Each patient underwent FLT PET imaging as well as bone marrow examination (gold standard). In addition, semi-quantitative (SUVmax and SUVmean) measurements of FLT uptake in the liver, spleen, and Lspine, SUVmean, as well as the Total Lesion Glycolysis (TLG) of the Lspine were performed. Results from the two patient cohorts were compared using = Kruskal-Wallis statistical test. A P-value of <.05 is considered to be statistically significant. Results: The differences in FLT SUVmax and SUVmean measurements in the three organs (liver, spleen, and LSpine) between the ET and Pre-PMF patients were not statistically significant (P > .05). In contrast, TLG measurements in the LSpine were statistically different (P = .013), and therefore, compared to gold standard bone marrow results, TLG can separate ET and Pre-PMF patients. Conclusion: This study is a proof of concept about the potential to discriminate between ET and pre-PMF patients in a non-invasive way. TLG of the LSpine in FLT PET images is a potential quantitative parameter to distinguish between ET and pre-PMF patients.

Aberrant Acquisition of T-cell Associated Markers in Plasma Cell Neoplasms: An Aggressive Disease with Extramedullary Involvement and Very Short Survival.

BACKGROUND: Plasma cell neoplasms can show aberrant expression of different lineage-related antigens; however, co-expression of T-cell-associated markers on malignant plasma cells is extremely rare. MATERIAL AND METHODS: This report describes clinicopathologic characteristics of three myeloma patients with emergent plasmablastic morphology and aberrant acquisition of T-cell-associated markers diagnosed in our center. An extensive literature search for similar cases was conducted, and the relevant pathologic, clinical, and prognostic characteristics were summarized. RESULTS: A total of 22 cases of plasma cell neoplasm (including the three cases reported here) showed aberrant co-expression of T-cell markers. We found an evident association between aberrant expression of T-cell markers on malignant plasma cells and extramedullary involvement, aggressive morphologic features, high proliferative index ki67 >90%, aggressive clinical course, an adverse outcome, and short survival. DISCUSSION & CONCLUSION: Due to the rarity of this aberrant phenotype and scarcity of the published data, the precise causative mechanism and its clinical implications have not yet been elucidated.

A study of 18F-FLT positron emission tomography/computed tomography imaging in cases of prefibrotic/early primary myelofibrosis and essential thrombocythemia.

The objectives of this research project are to study in patients with primary myelofibrosis (PMF) and Essential Thrombocythemia (ET); (1) the uptake patterns of FLT-PET (FLT-PET) and its value in diagnosing, staging, and treatment response monitoring of malignant hematopoiesis, (2) compare imaging findings from FLT-PET with bone marrow biopsy (standard of care), and (3) associate FLT-PET uptake patterns with genetic makeup such as JAK2 (Janus kinase 2), CALR (Calreticulin), MPL (myeloproliferative leukemia protein), Triple negative disease, and allele burden.This trial is registered in ClinicalTrials.gov with number NCT03116542. Protocol version: Mar 2017.

Subcutaneous Masses as an Unusual Manifestation of Relapse in a Case of Atypical Chronic Lymphocytic Leukemia with Prolymphocytoid Transformation and Complex Karyotype: A Diagnostic Dilemma and Treatment Challenge.

BACKGROUND Most patients with chronic lymphocytic leukemia (CLL) are asymptomatic at diagnosis, but 10% present with B symptoms. Most patients have palpable lymphadenopathy, while 20-50% of the patients have hepatosplenomegaly. Cutaneous infiltrations in patients with CLL can be localized or generalized in the form of erythematous papules, plaques, nodules and, ulceration, which is uncommon. CASE REPORT We present the case of a 71-year-old man diagnosed with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) with atypical immunophenotype and increased prolymphocytes (CLL/PLL), which was treated initially after white blood counts (WBC) doubling with Bendamustine and Rituximab for 6 cycles, and achieved complete remission. The patient relapsed after 6 months of completion of treatment, with multiple large subcutaneous masses, proved to be infiltration with the same atypical CLL/SLL on tissue biopsy, with pathologic features indicating disease progression. The lack of similar reported cases, and the aggressiveness of the tumor clinically and histopathologically, resulted in the decision to treat with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone (R-CHOP) as a case of aggressive lymphoma, with complete remission clinically and radiologically. CONCLUSIONS We present a rare case of subcutaneous extramedullary masses of atypical CLL/SLL. The high proliferation index (Ki-67) and the increase of large cells are suggestive of aggressive progression of the disease; however, no frank features of Richter's transformation were noted. Based on this and because of the unusual aggressive-looking skin masses, the panel decided to treat the patient with R-CHOP. The impact of this presentation on the prognosis of the disease is not clear. To date, our patient has responded well to treatment with R-CHOP, with complete remission of the subcutaneous masses and on PET scan, but further follow-up is needed.

Locally Aggressive Giant Cell Tumor of Bone With Pulmonary Distant Metastasis and Extrapulmonary Seeding in Pregnancy.

Giant cell tumor of bone (GCTB) is a locally aggressive benign neoplasm that is associated with a large biological spectrum ranging from latent benign to highly recurrent and occasionally metastatic tumor. In this article, we present a case of a 26-year-old woman who presented with swelling at the left lower ribs during pregnancy. Surgical excision was done, and histopathology showed tumor with features consistent with GCTB. MRI preformed after delivery revealed recurrence of the mass with extensive growth reaching 17 cm with two subcutaneous satellite nodules in the adjacent abdominal wall. positron emission tomography-computed tomography (PET-CT) scan revealed bilateral fluorodeoxyglucose (FDG)-avid lung nodules. Surgical resection was done, and histopathology showed no evidence of malignant transformation. Few months later, the tumor recurred again, with peritoneal deposits. The patient underwent wide massive resection of the recurrent mass and then started on denosumab therapy. Molecular analysis of the tumor detected H3F3A G34W mutation with no copy number alterations. We are presenting this case of GCTB with pulmonary distant metastasis and extrapulmonary seeding to upsurge awareness among clinicians about the possible extreme aggressive biological behavior of GCTB that can mimic the presentation of malignant bone tumor and also to discuss the possible predictive factors of such aggressive behavior.

The Role of Integrated Positron Emission Tomography/Computed Tomography (PET/CT) and Bone Marrow Examination in Staging Large B-Cell Lymphoma.

INTRODUCTION: In the era of routine use of positron emission tomography/computed tomography (PET/CT) for staging, it is not yet clear whether PET/CT can replace bone marrow biopsy for the assessment of bone marrow involvement in large B-cell lymphoma. OBJECTIVES: To compare the clinical utility of bone marrow biopsy and PET/CT scanning in the staging of large B-cell lymphoma. METHODS: This was a retrospective analysis of all patients who presented to single center over a 4-year period with large B-cell lymphoma who had concurrent PET/CT and bone marrow biopsy performed in the assessment and staging of the lymphoma. RESULTS: Out of 89 patients, 24 had bone marrow involvement either by PET/CT, by bone marrow biopsy, or by both. Bone marrow biopsy identified 12 patients (sensitivity 50%, specificity 100%, negative predictive value 84%), whereas PET/CT identified 23 patients (sensitivity 96%, specificity 100%, negative predictive value 98%). No patients were upstaged by the bone marrow biopsy result, and no patients had their treatment plan changed based on the bone marrow biopsy result. CONCLUSION: The results show that PET-CT is more sensitive and has better negative predictive value than bone marrow biopsy. This suggests that PET-CT could replace bone marrow biopsy in detecting bone marrow involvement for staging of large B-cell lymphoma.

IgG4-related disease mimicking pancreatic cancer: Case report and review of the literature.

INTRODUCTION: Most patients with pancreatic masses pose a diagnostic challenge when a benign lesion is suspected, and often, resection is needed before a benign diagnosis is confirmed. PRESENTATION OF CASE: A 57 years old male patient presented with a pancreatic head mass, obstructive jaundice and submandibular lymph node enlargement. He also had a history of recurrent eye pain and redness, skin lesions, and benign prostatic hypertrophy. MRI showed a pancreatic head mass with double duct sign, aortic thickening, bilateral renal lesions, diffuse lymph node enlargement, and prostatic enlargement. FDG-PET/CT demonstrated abnormal uptake corresponding to the MRI lesions, and there were elevated IgG4 levels on blood investigations. Biopsy of an inguinal lymph node revealed infiltrates with IgG4 plasma cells, consistent with the diagnosis of IgG4 disease. The patient was treated with IV steroids and showed significant improvement. DISCUSSION: IgG4 related disease is a rare entity that is characterized by lesions that show heavy infiltration with IgG4 positive plasma cells, storiform fibrosis, and obliterative phlebitis. The pancreas is the most commonly involved organ, but several other organ systems are involved, and this helps in clinical suspicion of the diagnosis. A biopsy from any easily accessible site that shows the characteristic histological features is sufficient for diagnosis. Patients respond quickly to steroids, but recurrence is frequent. CONCLUSION: IgG4 related disease is a rare cause of pancreatic tumorous lesions that need a high index of suspicion for diagnosis and should be differentiated from pancreatic neoplastic lesions.

Rare occurrence of metastasis from lung cancer to the anus: case report and review of the literature.

BACKGROUND: Anal metastases from lung cancer are infrequent, and there are only 10 published cases. Life expectancy is no longer than 1 year after diagnosis because of the typically advanced stage of disease. Treatment, which is typically inefficient, is administered with the intent to cure or avoid local complications. CASE PRESENTATION: We report a case of a patient with non-small cell lung cancer presenting with perianal metastasis mimicking an abscess. CONCLUSIONS: Because perianal masses may be misdiagnosed, patients with lung and other cancers should be evaluated for metastatic disease.

Tubercular Meningitis and Lymphadenitis Mimicking a Relapse of Burkitt's Lymphoma on (18)F-FDG-PET/CT: A Case Report.

Tuberculosis (TB) can present with various forms and can occasionally be mistaken for malignancy. Hereby, we report a 53-year-old man diagnosed and treated for Burkitt's lymphoma in 2009 who achieved a complete remission confirmed by a computed tomography (CT) scan. During the follow-up 2 years later, he complained of left hip pain that warranted investigation with magnetic resonance imaging and whole-body (18)F-fludeoxyglucose-positron emission tomography (FDG-PET)/CT which showed a benign lesion in the left hip associated with multiple lymph nodes in the chest and abdomen not amenable for biopsy. A follow-up PET/CT scan a few months later showed intense tracer uptake in the lymph nodes with size progression and appearance of new lymph nodes suspicious of lymphoma relapse. The patient was asymptomatic, and all investigations including viral and connective tissue disease studies were negative. Also the tuberculin skin test and QuantiFERON were negative. Lymph node biopsy was planned; however, the patient presented a few days earlier with fever, headache and photophobia. Cerebrospinal fluid (CSF) examination confirmed meningitis with lymphocytic pleocytosis and elevated protein. The CSF Gram stain, culture, viral and acid-fast bacilli were negative. CSF flow cytometry and cytopathology confirmed polyclonal lymphocytosis and suggested reactive causes. CSF TB culture grew Mycobacterium tuberculosis. Mediastinal lymph node biopsy also confirmed TB lymphadenitis. Four antituberculosis drugs were started. One year later, a PET/CT scan showed regression of all the involved lymph nodes. This case highlights the importance of excluding TB in patients with suspected malignancy, especially if they belong to endemic regions, and the increasing role of (18)F-FDG-PET/CT in the early detection of extrapulmonary TB.

Rapid response to and long-term effectiveness of anti-CD20 antibody in conventional therapy resistant Graves' orbitopathy: A five-year follow-up study.

Abstract The aim of this investigations was to study the effectiveness of anti-CD20 antibody therapy in Graves' orbitopathy (GO) resistant to glucocorticoids. Five patients were entered in the study. The protocol required no improvement of orbital status after a recent course of glucocorticoids. Activity of GO was confirmed by three independent techniques: clinical activity score (CAS), (99m)Tc-labeled diethylene triamine pentaacetic acid ((99m)Tc DTPA) single photon emission computed tomography and magnetic resonance imaging. Rituximab (RTX) was given as weekly infusions of 375 mg/m(2) body surface area for four weeks. The mean follow-up period was 67 (range 58-81) months. Improvement of GO has been observed in all patients: CAS before therapy was 6.5 ± 1.7 and decreased to 3.4 ± 1.6 by one month (p < 0.05) and remained unchanged (3.2 ± 1.7) at 12 months. No further CAS change, in either direction, was detected during the yearly follow-up visits. The mean DTPA uptake before therapy was 16.52 ± 4.51 MBq/cm(3) and decreased to 11.97 ± 2.36 MBq/cm(3) at one year (p < 0.002). The mean of T2 relaxation times before and one year after therapy were 96.91 ± 17.61 ms and 84.29 ± 9.41 ms, respectively (p < 0.001). The mean serum TSH receptor antibody (TRAb) levels before therapy, at the one month and one year control visits were 7.4 ± 3.4 U/L, 5.6 ± 4.5 U/L and 1.7 ± 1.5 U/L, respectively (p < 0.004). No correlation between changes of TRAb and activity parameters has been found. Anti-CD20 treatment seems to influence positively the clinical course of GO, and this effect seems to be stable for five years. To our knowledge, this is the longest published follow-up of RTX treatment in GO.

The impact of 99mTc-DTPA orbital SPECT in patient selection for external radiation therapy in Graves' ophthalmopathy.

OBJECTIVE: In Graves' ophthalmopathy (GO), there is a demand to differentiate the immunologically active disease state, when immunosuppressive therapy is necessary, from the inactive state, when the patient would not benefit from it. We measured the inflammatory activity in the retrobulbar region using Tc-diethylene triamine pentaacetic acid (DTPA) SPECT before and after external radiation to determine whether this method is suitable for predicting the effectiveness of this therapy. MATERIALS AND METHODS: Thirty-two patients with suspected active GO were involved in this retrospective study. The initial image, DTPA uptake value (UV) and its change after therapy were assessed to monitor the effect of the therapy and investigate whether a pretreatment scan is capable of predicting the outcome. RESULTS: Depending on the change in DTPA UV after radiotherapy, three patient groups were formed: decreased, increased or minimally changed (less than 1×10 injected dose (ID)/ml). The initial DTPA UVs of these groups were significantly different (P<0.001). Improvement was observed mainly in patients with higher initial values. When comparing the groups with low (<12×10 ID/ml) versus high (≥12×10 ID/ml) initial uptake, an unexpected increase was observed in the first group after therapy (mean: +2.89±2.66×10 ID/ml), whereas the average change in the DTPA UV was negative in the latter group as anticipated (-2.24±4.47×10 ID/ml, P<0.00001). CONCLUSION: We found that in GO patients a high DTPA UV may predict the response to orbital radiation therapy. DTPA orbital SPECT may be a suitable technique for the selection of GO patients for radiation therapy.

Simultaneous dacryocystography and dacryoscintigraphy using SPECT/CT in the diagnosis of nasolacrimal duct obstruction.

Epiphora, an abnormal overflow of tears, is commonly caused by tear drainage system anomalies including nasolacrimal duct obstruction. To assess morphologic abnormalities, dacryocystography by CT is used when CT contrast material is syringed into the lacrimal drainage system. To evaluate the function of the system, dacryoscintigraphy is the most readily available noninvasive method. In the case presented, a 43-year-old man was referred to our clinic with an 8 months' history of indefinite left side epiphora. After performing dacryoscintigraphy, we acquired SPECT/CT images during dacryocystography by CT to establish the surgical indication.